title: Central nervous system angiitis: a series of 31 patients.
abstract: Central nervous system (CNS) angiitis is a rare inflammatory disorder. To date, clinical data are lacking and treatment remains a matter of debate. The aim of this study is to analyse the main characteristics, response to therapy and outcome of 32 patients with CNS angiitis. Single-centre retrospective study in a tertiary centre was made. Diagnosis of CNS angiitis was made by cerebral angiography and/or magnetic resonance angiography and/or CNS biopsy. The main features and outcomes of primary and secondary CNS angiitis were compared and predictive factors of a favourable outcome were searched. Thirty-one patients (median age 45 Q1-Q3 37-54) sex ratio F/M 2.1) were included. Main clinical features were hemiparesis (35.5 %) and headache (29 %). The median CSF protein level was 0.64(0.52-0.81) g/L and was superior to 1 g/L in six cases. CNS magnetic resonance (MR) imaging findings were most frequently ischemic (96.8 %), bilateral (83.9 %), multiple (87.1 %) and supratentorial (96.8 %). The MR angiography was abnormal in all cases. Among the 31 patients in the study, 19 (61.3 %) were diagnosed with primary CNS angiitis. Systemic lupus erythematosus (n = 6) and vasculitis (n = 4) were the most frequent aetiologies of secondary CNS angiitis. No difference was evidenced between primary and secondary CNS angiitis. Steroids were administered in 79.2 % of treated patients and combined with immunosuppressants in 79.2 % of cases. Eight cases of CNS angiitis relapse were noted. CNS angiitis remains a severe illness. Treatment often associated steroids and immunosuppressants, and diagnosis delay is significantly associated with a poorer prognosis.
authors.affiliation: Service de Médecine Interne II, AP-HP, Hôpital Pitié-Salpêtrière, Paris, 75013, France.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Geri
authors.forename: David
authors.initials: D
authors.lastname: Saadoun
authors.forename: Rémy
authors.initials: R
authors.lastname: Guillevin
authors.forename: Sophie
authors.initials: S
authors.lastname: Crozier
authors.forename: Catherine
authors.initials: C
authors.lastname: Lubetzki
authors.forename: Karima
authors.initials: K
authors.lastname: Mokhtari
authors.forename: Zahir
authors.initials: Z
authors.lastname: Amoura
authors.forename: Bertrand
authors.initials: B
authors.lastname: Wechsler
authors.forename: Du
authors.initials: D
authors.lastname: Le Boutin
authors.forename: Nathalie
authors.initials: N
authors.lastname: Costedoat-Chalumeau
authors.forename: Yves
authors.initials: Y
authors.lastname: Samson
authors.forename: Patrice
authors.initials: P
authors.lastname: Cacoub
mesh_terms: D000328:Adult; D001706:Biopsy; D002533:Cerebral Angiography; D005260:Female; D006261:Headache / Q000150:complications; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D018810:Magnetic Resonance Angiography; D008297:Male; D008875:Middle Aged; D010291:Paresis / Q000150:complications; D012189:Retrospective Studies; D012307:Risk Factors; D013256:Steroids / Q000008:administration & dosage; D016896:Treatment Outcome; D020293:Vasculitis, Central Nervous System / Q000134:cerebrospinal fluid / Q000175:diagnosis* / Q000473:pathology
chemical_list: D007166:Immunosuppressive Agents; D013256:Steroids